R2029
Monoclonal Anti-Rapsyn antibody produced in mouse
1 mg/mL, clone 1234, purified immunoglobulin, buffered aqueous solution
别名:
Anti-CMS11, Anti-CMS4C, Anti-FADS, Anti-FADS2, Anti-RAPSYN, Anti-RNF205
生物来源
mouse
质量水平
偶联物
unconjugated
抗体形式
purified immunoglobulin
抗体产品类型
primary antibodies
克隆
1234, monoclonal
表单
buffered aqueous solution
种属反应性
amphibian, chicken, mouse, rat, fish
浓度
1 mg/mL
技术
western blot: 0.5 μg/mL using Torpedo californica electrocyte cell extracts
同位素/亚型
IgG1
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
mouse ... Rapsn(19400)
rat ... Rapsn(362161)
一般描述
The receptor-associated protein of the synapse (RAPSYN) is a peripheral membrane protein located in the skeletal muscles. It is co-localized along with nicotinic acetylcholine receptors (nAChRs) at the postsynaptic membrane due to the influence of neural agrin. Rapsyn consists of a myristoylated N-terminal, seven putative tetratricopeptide repeats, a coiled-coil domain, the RING-H2 domain, and the consensus sequence for phosphorylation.
免疫原
whole purified rapsyn from Torpedo californica electric organ postsynaptic membrane.
应用
Monoclonal Anti-Rapsyn antibody produced in mouse has been used in western blotting (0.5 μg/ml ) and immunocytochemistry (1:200).
生化/生理作用
The receptor-associated protein of the synapse (RAPSYN) participates in assembling the structure of the nicotinic postsynaptic membrane. It is required for the functioning of the neuromuscular junction. Mutations in the RAPSN gene are associated with endplate acetylcholine-receptor deficiency and Myasthenic syndrome.
外形
Solution in phosphate buffered saline containing 1.0 mg/mL BSA and 0.05% sodium azide.
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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储存分类代码
10 - Combustible liquids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
Marianna Rodova et al.
Molecular and cellular biology, 24(16), 7188-7196 (2004-07-30)
Rapsyn is a synapse-specific protein that is required for clustering acetylcholine receptors at the neuromuscular junction. Analysis of the rapsyn promoter revealed a consensus site for the transcription factor Kaiso within a region that is mutated in a subset of
M Bartoli et al.
The Journal of biological chemistry, 276(27), 24911-24917 (2001-05-09)
Rapsyn, a peripheral membrane protein of skeletal muscle, is necessary for the formation of the highly organized structure of the vertebrate neuromuscular junction. For mice lacking rapsyn, there is a failure of postsynaptic specialization characterized by an absence of nicotinic
M K Ramarao et al.
The Journal of biological chemistry, 276(10), 7475-7483 (2000-11-23)
Rapsyn, a 43-kDa peripheral membrane protein of skeletal muscle, is essential for clustering nicotinic acetylcholine receptors (nAChR) in the postsynaptic membrane. Previous studies with rapsyn NH(2)-terminal fragments fused to green fluorescent protein, expressed in 293T cells along with nAChRs, establish
Rapsyn mutations in humans cause endplate acetylcholine-receptor deficiency and myasthenic syndrome.
Kinji Ohno et al.
American journal of human genetics, 70(4), 875-885 (2002-01-16)
Congenital myasthenic syndromes (CMSs) stem from genetic defects in endplate (EP)-specific presynaptic, synaptic, and postsynaptic proteins. The postsynaptic CMSs identified to date stem from a deficiency or kinetic abnormality of the acetylcholine receptor (AChR). All CMSs with a kinetic abnormality
Benoît Zuber et al.
Proceedings of the National Academy of Sciences of the United States of America, 110(26), 10622-10627 (2013-06-12)
The scaffolding protein at the neuromuscular junction, rapsyn, enables clustering of nicotinic acetylcholine receptors in high concentration and is critical for muscle function. Patients with insufficient receptor clustering suffer from muscle weakness. However, the detailed organization of the receptor-rapsyn network
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